For Rachel and Stacey Wood, every breath is precious.
Diagnosed with cystic fibrosis when she was only five months old, Rachel, now 9, looks like a normal, healthy girl: straight, blond hair flying behind her shoulders as she plays with Auggie, the family’s dog, as he runs around in playful circles.
Stacey looks at her daughter and sighs. “She’s just like a normal kid except she’s got this regime of treatments she goes through daily to keep her healthy,” she said.
Cystic fibrosis is a degenerative disease affecting the lungs and pancreas by coating or filling them with a thick, sticky mucus that is not thinned naturally due to a lack of certain enzymes, Wood said. “Often, kids are diagnosed because they can’t gain weight. The mucus in her lungs affects the transfer of salt from her food into her bloodstream, so she can’t gain weight, and it’s the same with her pancreas,” she said.
When Rachel was only five months old, she picked up a cough from her twin brother, Kevin. Within a few weeks, Kevin was fine again but Rachel couldn’t shake it, Wood said. “She only weighed about eight pounds at five months, we knew something was wrong. There’s no history of CF in either my or my husband’s families. We had nothing to compare it with.”
“I had to stay in the hospital for 10 days and 10 nights,” Rachel said, taking her attention off Auggie for a moment.
Initially, Wood was told to try a different formula for Rachel, but the cough persisted. Her doctors thought it was whooping cough or some other respiratory illness, but after several days, a sweat test was performed on Rachel, she said.
“She came back negative for CF on the first test, but she didn’t get better, so we went to New York City for a second test, which came back positive,” she said. “Ten days of antibiotics later, she’s been fine. That’s that only time she’s been overnight in a hospital.”
There are several things Rachel has to do every day to keep her lungs as clear as possible.
“There’s a vest I have to wear,” she said. “You put it on over your head and plug it into a machine and turn it on and it shakes you up.”
“The vest shakes you to dislodge the mucus in your lungs,” Wood said. “We do that for six minutes on each of the three settings twice a day.”
There’s also a nebulizer that Rachel calls “The smoker” which she spends several minutes breathing into every day.
“I also take the Advair disk every day,” Rachel said, an inhalant that works to keep her airway open.
She takes two medicines, Vithromax and Bactrium, as preventative medicines daily too, Wood said.
“If she gets a cold, we’re on our way to Johns Hopkins hospital to get her medicine,” she said.
UNFORTUNATELY, HER BODY may become accustomed to some of the medicine Rachel can take when she has a cold or to keep her breathing normally, which would render it useless in the fight against suffocation.
“It’s a catch-22,” Wood said. “Do you leave her on the medication when she’s not sick to keep her from getting sick or do you take her off it to keep her from getting used to it? I don’t know, it’s a really difficult decision.”
Wood said she is encouraged by the discovery of the gene linked to cystic fibrosis since Rachel was born, but said there is still no cure in sight.
“There are some kids that die in infancy, some die in their teenage years, some live to be 60 and healthy up until the end,” she said. “The doctors can’t tell you how long it can take.”
Currently, the average life expectancy is a little over 34 years, she said.
The Wood family will be participating in the second annual Great Strides walk-a-thon in Great Falls this Sunday, May 22, starting at 1 p.m. in the Wachovia parking lot on Walker Road. Last year, the walk raised over $11,000 for cystic fibrosis research through the Cystic Fibrosis Foundation.
“In order to advance the research, we have to work as quickly as possible,”
said Julie Puzzo, executive director of the Cystic Fibrosis Foundation in Bethesda. “Almost all of the money we raise goes directly into research. It’s very targeted and organized.”
Only 30,000 people in the United States are affected by cystic fibrosis, which means some of the larger pharmaceutical labs are not willing to spend hundreds of thousands or millions of dollars into looking for a stronger treatment or cure.
“We’re very careful about the amount of money we spend on research, so we tend to go for smaller companies that can dedicate more time to it,” she said. Over 90 cents from every dollar raised goes directly to research, she added.
The Great Strides walk is one of the few situations where children and families touched by cystic fibrosis can come together to share stories and have support, she said.
“It’s difficult for C.F. patients,” she said. “You can’t have a camp for kids with C.F. because they could pass infections to each other. This walk is one of the only chances these kids have to all be together. It’s safe because they’re in an open space, and there shouldn’t be much chance for infections to spread from one to another.”
Currently there are 21 drugs being investigated by small laboratories that have been given the green light for further study, Wood said.
“Last year was the first year there was more research than we had money for,” she said. “That’s great, but at the same time, we can’t afford to wait. We need to win this race.”
THIS IS THE third time the Woods have walked in the Great Strides event. Last year, over $170,00 was raised from the walk. This year, the nationally held walk is expected to bring in a total of $28 million nationally, with $900,000 coming from the Washington, D.C., area, Puzzo said.
“The Woods have been really great. They’ve tried to go with the mindset that they can’t [find the cure] themselves, but they can do a lot to raise money to help,” she said. “Rachel is one of the most resilient children I’ve ever met, and I think most of the kids with C.F. are much older than their years because they have to be. We want them to be able to be kids,” she said.
In addition to the walk, the Great Falls community has held several events prior to the walk to raise money for research.
“The community has done so much to rally around Rachel,” Puzzo said. “There’s been a lot of fun events, like a coffee hour, a margarita night, donut sales and pizza lunches, all these things leading up to the walk so the actual event is really exciting.”
Rachel does not think much about her twin brother being healthy while she has a disease that may some day take her life.
“My mom thinks about it more than I do,” she said. “I don’t really think about it much at all. I don’t really care.”
“Rachel is lucky. She doesn’t get held back from anything,” Wood said. “She goes to regular school, she plays a lot of sports, she’s very active and plays with her three brothers all the time. She leads a normal life.”
Any activity that makes her lungs work is good for her, Wood said. That Rachel is so active may help keep her as healthy as possible.
If a cure is found, however, the damage done to the lungs may be irreversible.
“Just by having cystic fibrosis, a person’s lung capacity is decreased something like 1 or 2 percent per year,” she said.
Watching her daughter run, laughing, Woods remembers that everything is not as it seems.
“Even if you think you’re healthy, you’ve got this disease lurking in your chest,” she said. “It’s there. It’s always there. And I don’t want it to be there anymore.”
Kevin is a carrier for cystic fibrosis, but the Woods’ other two sons, Ian, 12, and Jason, 10, are not, she said. “There are some families that have two or more children with cystic fibrosis, and it’s so much harder,” she said.
Cystic fibrosis can be diagnosed with an in-utero test, part of a standard pregnancy exam, she said.
For now, Wood looks to the future with optimism, hoping that further study of the gene linked to cystic fibrosis is a stepping stone on the way to a cure.
“She’s a very strong person,” she said of her youngest child and only daughter. “She’s becoming much more aware of her disease, and she’s asking more and more questions.”
The third annual Great Falls Great Strides walk will begin with registration at the Wachovia Bank branch on Walker Road, across the road from Safeway and near the Village Center shopping area, at noon, with the 5K walk or run beginning at 1 p.m. Donations can be made to Rachel’s Hope, care of the Cystic Fibrosis Foundation, 6917 Arlington Road, Bethesda, 20814. More information on cystic fibrosis is available at www.cff.org.